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Sebastian Thaler Manfred Zierhut Centre of Ophthalmology University of Tuebingen, Germany

Whipple´s Disease. Sebastian Thaler Manfred Zierhut Centre of Ophthalmology University of Tuebingen, Germany. First Presentation – Ocular History November 2006. 56 year old white German man OU: persisting vitreous inflammation since 2 months

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Sebastian Thaler Manfred Zierhut Centre of Ophthalmology University of Tuebingen, Germany

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  1. Whipple´sDisease Sebastian Thaler Manfred Zierhut CentreofOphthalmology University of Tuebingen, Germany

  2. First Presentation – OcularHistory November 2006 • 56 yearoldwhite German man • OU: persistingvitreousinflammationsince 2 months • Complains:reddening, foreignbodysensations, pressurefeeling • treatmentPrednisolone (10 mg) • topicalcorticosteroids 5x/day

  3. First Presentation – OcularHistory November 2006 • „intermediate uveitis“ since 2/2003 • diagnostic ppV • OD (2x) (12/03 and 4/04) nomalignancy • OS (1x) (3/05) • last recurrence 2/06 • improvement after 50 mg ofprednisolone

  4. First Presentation – OcularHistory November 2006 • last recurrences (2 and 6/06) • goodresponsetosystemiccorticosteroids • recurrences after reduction

  5. First Presentation – General History November 2006 • arthritis – non-steroidalantiphlogistics • noothercomplains

  6. First Presentation – OcularExamination November 2006 • VA: 0.1/0.5 • IOP: 20/ 18 mmHg • OD: AC-cells 3+, snowflake-like particles in the AC, irishyperemia, pseudophacos, funduswithoutdetails • OS: AC cells 1+, cataract, funduswithoutsignsofinflammation

  7. First Presentation – Anterior Segment OD OS

  8. First Presentation – AnteriorSegment OD Thaler et al. Int. J. of Infectious Diseases 2010

  9. First Presentation – PreviousInvestigations • CT-brain: unremarkable • chest X-ray: unremarkable • lab: ESR 30/60, Ig-A1 und A2 upperlimit. TSH mildlyelevated • serology: Lyme´sdisease, syphilis, bartonella: all negativ • neurologicallynosignsofinflammation

  10. First presentation – Diagnostic ppV • nodetectionofbacteria, nofungus • vitreal histology • purulent unspecificinflammation • notypicalcells, nosignsofmalignancy • molecularbiology: nosignsoflymphoma

  11. First Diagnosis • suggestive forlowgrade endophthalmitis • removalof IOL • in addition intravitreal antibiotics

  12. Follow Up – After 1 MonthRe-ppV • nodirectdetectionofbacteriaorfungus • PCR: Tropherymawhippleipositiv . Thaler et al. Int. J. of Infectious Diseases 2010

  13. Final Diagnosis • Uveitis due toWhipple´sDisease • based on • clinicalfindings • positive PCR fromthevitreous

  14. Diagnostics – After 2 MonthsJanuary 2007 • biopsyofthesmallintestine • detectionofTropherymawhipplei • blood • detectionofTropherymawhipplei • spinal puncture • nodetectionofTropherymawhipplei • endoscopy: • antrumofthestomach with spottedmucosalatrophy

  15. Treatment Begin January 2007 • intravitreal Ceftriaxonfor 2 weeks followedby • oral Trimethoprim with Sulfamethoxazolfor 1 year • planned: secondarylensimplantation

  16. Final Follow Up – After 5 MonthsMarch 2007 • VA OD: 0.2, OS: 0.67 • no signs of intraocular inflammation • regular controls necessary because • recurrences 11 years after stop of antibiotic treatment reported

  17. Final Follow Up – After 6 MonthsApril 2007 • re-biopsyfromthesmallintestine: • PCR negativ

  18. Whipple´sDisease – Clinical SymptomsIntestinal • diarrhea • abdominal pain • malabsoption, leadingtoanemia,hypoproteinemiaandhypovitaminosis • weightloss

  19. Whipple´sDisease – Clinical SymptomsExtraintestinal • arthralgia(oftenyearsbefore intestinal symptoms) • erythemanodosum • neurologicalsymptomes (dementia, Parkinson´sdisease, headach) • sec. ocularinvolvement: ophthalmoplegia, nystagmus • chroniccought (DD TB) • heartinsufficiency, anginapectoris • rarelyprimaryUveitis (<3%)

  20. Whipple´sDisease - Detection • PAS-staining • PAS-positive SPC-Zellen: siccleparticlescontainingcells • culture: verydifficult

  21. Differential Diagnosis • intraocularlymphoma • ischemicophthalmopathy • low grade endophthalmitis • sarcoidosis • TB

  22. Whipple´sDisease - Therapy • systemicantibioticsfor 1 year • lethalifwithouttreatment • in caseofocularinvolvement • Trimethoprim+ Sulfamethoxazol • Doxycyclin + Rifampicin • Chloramphenicol • Ceftriaxoni.v.occ. forshort time

  23. Conclusion • rarely intermediate uveitiscanbecausedbyWhipple´sDisease • detectionofTropherymawhippleicanbedonefrom • vitreous, IOL and pars plana precipitates • blood • smallintestine • therapyconsistsofantibioticsfor 1 year

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